SUCLG1
This is the approved gene symbol for the gene encoding the alpha subunit of the mitochondrial Krebs-cycle enzyme Succinate-CoA ligase (Succinyl-CoA synthetase; EC6.2.1.4), which is being referred to also as SUCLA1 [Succinyl-CoA synthetase-alpha] (abbr. SCS-alpha) (James et al, 1997).
The enzyme produces succinyl-CoA by catalyzing the ligation of succinate and CoA. This process depends on ATP or GTP, and the nature of the beta subunit determines the nucleotide specificity.
A deficiency in this enzyme leads to mitochondrial dysfunction (Mitochondrial DNA depletion syndrome 9) (Ostergaard et al, 2007; Carrozzo et al, 2016).
Role as moonlighting protein
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